Cases reported "Cystadenocarcinoma"

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1/9. Biliary cystadenocarcinoma of the liver: the need for complete resection.

    We report on a patient with biliary cystadenocarcinoma and review 112 previously published cases of this rare cystic hepatic neoplasm. This tumour mainly occurs in women at a ratio of 62% (female) to 38% (male), and at an average age of 56.2 years (range 18-88 years). The origin of these neoplasms is intrahepatic in 97% of cases and extrahepatic in the remaining 3%. The clinical symptoms are nonspecific and are not distinctive from benign cystic liver lesions unless invasive growth of the tumour occurs or distant metastases are present. Sonography and computed tomography (CT), as well as magnetic resonance imaging (MRI) demonstrate the multilocular nature of the tumour with septal or mural nodules. Discrete soft tissue masses, thick and coarse calcifications and varying density on CT or intensity on MRI within the loculi are additional non-specific imaging findings. The best therapeutic result with a 5-year survival rate of 100% and a recurrence rate of only 13% was achieved by complete excision (n = 16). Surgical removal of the tumour by complete excision is, therefore, the treatment of choice for biliary cystadenocarcinomas.
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2/9. Potential clinical utility of CA-125 in responsive but persistent large-volume ovarian cancer following platinum-based chemotherapy.

    BACKGROUND: Despite the demonstrated clinical utility of the serum ca-125 antigen level in ovarian cancer, controversy exists regarding interpretation of "discordant" results between changes in this tumor marker and measurable disease masses. CASE: A patient with ovarian cancer cared for in the Gynecologic Cancer Program of the Cleveland Clinic Foundation receiving second-line single-agent carboplatin for recurrent disease demonstrated a major response in serum CA-125, but minimal shrinkage of a large painful abdominal mass. A laparotomy was performed both to define the nature of this mass and to attempt to relieve symptoms. The mass was found to be a large "inflamed pseudotumor with central necrosis." No viable tumors cells were found. CONCLUSION: This case represents an excellent example of the remarkably complex biology of malignant disease and suggests how evaluation of changes in CA-125 in women with ovarian cancer may be utilized in individual patients to develop optimal management plans.
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3/9. Oncocytic differentiation in intrahepatic biliary cystadenocarcinoma.

    An intrahepatic biliary cystadenocarcinoma in a 56-yr-old white man was characterized by pronounced oncocytic differentiation. Grossly the tumor was a well-demarcated cyst filled with numerous branching papillary fronds. Most tumor cells had abundant granular, intensely eosinophilic cytoplasm on light microscopic examination and large numbers of densely packed mitochondria by electron microscopy. Mucin-secreting cells were also present. The patient returned 20 mo after resection of the primary tumor with recurrent tumor in the liver and widely disseminated disease throughout the abdominal cavity, and he died 5 mo later. Although less differentiated, the recurrent tumor again contained greatly increased numbers of mitochondria. The partial loss of oncocytic differentiation in the evolution of the present case and the benign nature of purely oncocytic tumors suggest that in the presence of mixed histologic features the potential for tumor progression is primarily determined by the lesser differentiated or nononcocytic component. To the best of our knowledge, oncocytic differentiation has not been previously described in biliary neoplasia.
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4/9. Low-grade salivary duct carcinoma: description of 16 cases.

    Low-grade salivary duct carcinoma is a rare neoplasm. We report on 16 patients, with a median age of 64 years. All but one tumor arose from the parotid gland, including one tumor that arose in an intraparotid lymph node; one arose in the submandibular gland. Tumors consist of single to multiple dominant cysts, accompanied by adjacent intraductal proliferation. cysts are lined by small, multilayered, proliferating, bland ductal cells with finely dispersed chromatin and small nucleoli. Separate, smaller ductal structures are variably filled by proliferating ductal epithelium with cribriform, micropapillary, and solid areas. The overall appearance is very similar to breast atypical ductal hyperplasia and low-grade ductal carcinoma in situ. Foci of definitive stromal invasion were seen in four tumors. Two tumors demonstrated transition from low- to intermediate- or high-grade cytology, with scattered mitotic figures and focal necrosis. S-100 revealed diffuse strong expression in all 9 cases studied. Myoepithelial markers (calponin) highlighted supportive myoepithelial cells rimming the cystic spaces, confirming the intraductal nature of most, or all, of six tumors studied. Nine tumors studied for Her2-neu antigen were uniformly negative. Follow-up was obtained on 13 of our 16 patients. All patients were disease-free after surgery 6 to 132 months (median 30 months). Low-grade salivary duct carcinoma is a low-grade neoplasm with an excellent prognosis; it may be treated by conservative but complete resection. Its resemblance to atypical breast ductal hyperplasia, or micropapillary/cribriform intraductal carcinoma, distinguishes it from high-grade salivary duct carcinoma, papillocystic acinic cell carcinoma, and cystadenocarcinoma.
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5/9. Pancreatic mucinous cystadenocarcinoma with pseudosarcomatous mural nodules. A report of a case with immunohistochemical study.

    A case of pancreatic mucinous cystadenocarcinoma (PMC) with two pseudosarcomatous mural nodules (PMN) is described. These nodules have not been previously described in this type of tumor. In ovarian mucinous tumors (OMT), similar nodules have been reported, the nature of which has been discussed in detail. Here the similarity between the tumor described here and ovarian tumors is stressed. The immunohistochemical study carried out disclosed in the nodules strong positive staining for vimentin and moderate positivity for keratin and epithelial membrane antigen. These findings, along with histologic details, favor the epithelial nature of the nodules. It was concluded that the nodules are foci of anaplastic carcinoma with high proliferative cell rate, which could explain the coexpression of vimentin and keratin.
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6/9. cystadenocarcinoma of the pancreas 9 years after a pancreatic cyst operation: report of a case.

    A patient case with cystadenocarcinoma of the pancreas in a middle-aged woman is described. Nine years prior to the pancreatic malignancy she underwent cystogastrostomy due to a pancreatic cyst presumed to be of non-neoplastic nature. The malignancy occurred at the site of the previous cystogastrostomy and was radically resected. Five years later the patient was well without signs of recurrence. The possibilities of incorrect primary diagnosis or development of a rare pancreatic malignancy in a previous cystogastrostomy are discussed.
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7/9. Cystic tumors mistaken for pancreatic pseudocysts.

    A small fraction of pancreatic cysts are neoplastic rather than inflammatory in origin. Failure to recognize the true nature of a neoplastic cyst will lead to an incorrect treatment strategy. This is a report of eight patients whose cystic neoplasms were misdiagnosed and maltreated. Five of the eight tumors proved to be malignant. Five were drained by anastomosis to a viscus and one by aspiration; drainage was recommended for the other two. Treatment by drainage led to complications (persistent painful gastric ulcer, infection in the cysts), growth of new cysts, no cures, but missed opportunities to cure cancer. Three patients with no metastases at first operation had metastatic spread to the liver, omentum, or lungs at reoperation. In three of the five cases initially treated by cystenterostomy (including one cancer), subsequent resection was possible and probably curative. One cystadenocarcinoma was watched for 3 years before apparently curative resection. Guidelines based on serum and cyst amylase levels, morphologic appearance, angiography, pancreatography, and biopsy are given for the purpose of differentiating inflammatory cysts from neoplastic cysts of the pancreas. confusion of these entities should not occur, but errors can often be corrected.
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8/9. Ovarian mucinous cystadenocarcinoma with mural nodules of anaplastic carcinoma: report of a case and review of the literature.

    Cases of ovarian mucinous cystadenocarcinoma with mural nodules of anaplastic carcinoma are very rare. We report on such a case and review the literature. The mural nodules in our case were made up mainly of anaplastic spindle cells and numerous osteoclast-like giant cells. They resembled sarcoma or sarcoma-like mural nodules under light microscopy. The anaplastic cells within the nodules were immunopositive for both cytokeratin and vimentin, and ultrastructurally they displayed desmosomes. This case reaffirms the value of immunohistochemistry and ultrastructural study to establish the nature of such nodules, and the importance of making such distinctions.
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9/9. Primary biliary cystadenocarcinoma perforating the duodenum and left intrahepatic biliary tree--mimicking a hydatid cyst.

    We report the case of a 76-year-old woman with biliary cystadenocarcinoma perforating the left biliary tree and exhibiting intra-tumoral gas bubbles resulting from invasion of the duodenum. The clinical history included subfebrile temperatures of 3 months duration, and pains associated with an abdominal mass in the right upper quadrant. blood tests showed leucocytosis, and radiological studies revealed the features of a partially calcified septated tumor with nodular components combined with multiple gas-fluid levels, mimicking an infected hydatid cyst. Intraoperative ultrasonography, cholangiography and frozen section histology were necessary to prove the malignant nature of this cystic tumor. Provided that complete resection with strict adherence to oncological precepts is possible, the prognosis of cystadenocarcinoma is better than in hepatocellular or cholangiocellular carcinoma.
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